February 08, 2016
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Oregon teen’s death linked to rare measles complication

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The Oregon Health Authority reported that the 2015 death of a boy aged 14 years was the result of subacute sclerosing panencephalitis, a rare but fatal complication of measles, according to a case study published in MMWR.

“This report demonstrates the devastating neurological decline and social impact from this rare complication of measles,” Juventila Liko, MD, at the public health division of the Oregon Health Authority, told Infectious Diseases in Children. “Measles is worth preventing.

When aged 8 months, the patient received the measles vaccine in the Philippines, the researchers wrote. He contracted measles, however, 4 months later while still living in the Philippines. After recovering, he remained well for 10 years.

The report said he developed neurodegenerative symptoms in 2012, including dropping utensils, struggling with homework, falling asleep during meals and while walking. He experienced increasingly frequent myoclonic jerks as well as involuntary hand and arm movements. Eventually, a pediatric neurologist was consulted. In the initial evaluation, no family history of neurologic disease was reported. The evaluation also included a lumbar puncture and an MRI of the brain, both considered unremarkable. The neurologist administered an electroencephalogram that was abnormal — with frequent, high-amplitude bifrontal slowing. As a result, the neurologist was unable to determine the cause of the patient’s condition.

In the following month, the patient was unable to sit still, Liko and colleagues wrote. He frequently fell, became aggressive and could no longer be tutored. He began to shuffle as he walked, and he began to walk on his toes, until eventually refusing to walk. He cried continuously and slept for longer periods. Although he was responsive, his speech declined, and eventually he could only say a few words. The patient also could no longer recognize his family members’ faces or voices. Ultimately, he became incontinent and unable to eat or drink.

In 2012, he was admitted to a tertiary care children’s hospital in Oregon, the researchers said. Upon admission, he showed abnormal movements in his arms and legs and was unresponsive to questions. The EEG during admission showed moderate, diffuse background slowing and disorganization, with multiple spikes and sharp waves, characteristic of subacute sclerosing panencephalitis (SSPE). His serum measles IgM was negative, but his serum measles IgG level was elevated at greater than 11 index value (IV), and his cerebrospinal fluid (CSF) measles IgG level was greater than 10 IV, which also is characteristic of SSPE.

At the CDC’s measles virus laboratory, the CSF measles IgG level was confirmed, along with a diagnosis of SSPE. The researchers wrote that during the 1989-1991 measles resurgence in the U.S., SSPE incidence was 4 to 11 cases per 100,000 measles cases. Because no specific therapy was available, the patient was discharged from the hospital after 14 days. He received 3 years of hospice care until his death in 2015.

According to the researchers, measles vaccination during early infancy has been associated with lower seroresponses than those of children vaccinated later. Thus, the CDC continues to recommend that the first dose of the measles, mumps and rubella vaccine be administered in children aged at least 1 year and the second dose between 4 and 6 years. However, when traveling abroad, where measles is more common, infants should receive one dose of the vaccine when aged 6 to 11 months and two additional doses, the first when the patient is aged 12 and 15 months and the second at least 28 days later.

“This case underscores the importance of maintaining high-population immunity, through routine administration of two doses of measles-containing vaccine to all eligible children,” Liko and colleagues wrote. “Widespread measles vaccination is associated with the near disappearance of SSPE in the United States.” – by Will Offit

Disclosure: The researchers report no relevant financial disclosures.